Academic Success Week 09 - GI Embryology & Nitrogen Metabolism
- 1.A 2 month old baby boy presents with a swollen umbilicus that has failed to heal properly. The umbilicus drains secretions and there is passage of fecal matter through the umbilicus at times. What is the most likely diagnosis?
- A.
Gastroschisis
- B.
Anal agenesis
- C.
Ileal diverticulum
- D.
Omphalocele
- E.
Intestinal volvulus
- 2.A five month old female infant presented at the pediatric unit due to yellow pigmentation of the skin which had developed on the 3rd day of life. Clinical examination showed jaundice with mild abdominal distention and hepatosplenomegaly and no ascites (fluid accumulation in abdominal cavity). The lungs and the heart were essentially normal. Stool was clay colored. Infection screen and hepatitis markers were negative. Bile salts and bile pigments were detected in the urine. What is the most likely diagnosis?
- A.
Duodenal stenosis
- B.
Pyloric stenosis
- C.
Extra hepatic biliary atresia
- D.
Neonatal hemochromatosis
- E.
Viral hepatitis
- 3.Periodontal ligaments are derived from
- A.
Ectoderm
- B.
Oral epithelium
- C.
Odontoblasts
- D.
Mesoderm
- E.
Neural crest
- 4.Which of the following events in the development of the abdominal cavity is affected by the rapid growth of the liver?
- A.
Formation of greater omentum
- B.
Formation of lesser sac
- C.
Physiological herniation of the midgut loop
- D.
Urorectal septum formation
- E.
Formation of pancreas
- 5.Prenatal ultrasound revealed polyhydramnios at 36 weeks, and at birth the infant had excessive fluids in its mouth and difficulty breathing. Which of the following is the most likely cause for these conditions?
- A.
Pyloric stenosis
- B.
Enterocystoma
- C.
Annular pancreas
- D.
Esophageal atresia with tracheoesophageal fistula
- E.
Meckel's diverticulum
- 6.The chorda tympani nerve, a derivative of the facial nerve, transmits taste fibers from the anterior two-thirds of the tongue to the brain. What is the best embryological reason for this anatomical pattern?
- A.
Contribution of the copula from pharyngeal arch II to the formation of the adult tongue
- B.
Contribution of the hypopharyngeal eminence from pharyngeal arch III to the formation of the adult tongue
- C.
A pretrematic branch from the nerve of pharyngeal arch II to arch I
- D.
A pretrematic branch from the nerve of pharyngeal arch III to arch II
- E.
A pretrematic branch from the nerve of pharyngeal arch IV to arch III
- 7.Meckel's diverticulum is a remnant of the
- A.
Ventral mesentery
- B.
Allantois
- C.
Lesser sac
- D.
Vitelline duct
- E.
Cloaca
- 8.Which of the following structures is derived from hindgut?
- A.
Liver
- B.
Ascending colon
- C.
Descending colon
- D.
Appendix
- E.
Cecum
- 9.Which of the following structures is derived from the midgut?
- A.
Liver
- B.
Descending colon
- C.
Appendix
- D.
Gallbladder
- E.
Spleen
- 10.Appendix is derived from the
- A.
Dorsal mesentery
- B.
Ventral mesentery
- C.
Foregut
- D.
Hindgut
- E.
Midgut
- 11.Which of the following structures can be found at the free margin of falciform ligament?
- A.
Portal triad
- B.
Median umbilical ligament
- C.
Superior mesenteric artery
- D.
Round ligament of the liver
- E.
Cystic duct
- 12.Which of the following amino acids is both essential and glucogenic?
- A.
Alanine (Ala, A)
- B.
Methionine (Met, M)
- C.
Leucine (Leu, L)
- D.
Proline (Pro, P)
- E.
Lysine (Lys, K)
- 13.K.L. was born in Ville de Saguenay (Quebec), birth was uneventful. However, she failed to thrive. Suddenly at the age of 5 month her condition deteriorated rapidly and she was transferred to an emergency room. Organ function tests indicated failure of liver and kidney, attending physicians noticed a strong cabbage-like smell originating from her. The most likely diagnosis is
- A.
Hyperlysinemia
- B.
Cystinuria
- C.
Methylmalonic aciduria
- D.
Tyrosinemia I
- E.
Glycine cleavage enzyme deficiency
- 14.The presence of phenylpyruvate and phenylketones in a child's blood are indications of the genetic disorder Phenylketonuria. How does this compound accumulate? This is a disorder of phenyalanine metabolism which results from:
- A.
Too little product formation of a metabolic pathway
- B.
Too much substrate accumulation for an enzymatic reaction
- C.
An undersupply of tyrosine for protein translation
- D.
An oversupply of homogentisic acid
- E.
Modification of blood proteins by metabolic acids
- 15.Coconut protein is biologically inferior because it is lacking many essential amino acids. In your efforts to make transgenic coconuts with protein of high biological value, you should definitely ensure that your coconut protein has an adequate amount of:
- A.
Glutamine
- B.
Arginine
- C.
Phenylalanine
- D.
Tyrosine
- E.
Serine
- 16.High homocysteine levels are associated with an increased risk of atherosclerosis. A deficiency of which of the following enzymes would result in high blood levels of homocysteine (and also methionine)?
- A.
Tyrosine hydroxylase
- B.
Cystathionine synthase
- C.
Threonine dehydratase
- D.
Glutathione reductase
- E.
L-amino acid oxidase
- 17.A negative nitrogen balance is most likely in:
- A.
A heavyweight boxer training for the world championship
- B.
A fast-growing teenager who eats 3500 calories of junk food every day
- C.
A political prisoner who went on a hunger strike
- D.
A woman who is 8 months pregnant
- E.
A patient who is recovering from successful cancer surgery and chemotherapy
- 18.A 2-week-old infant presents with vomiting, lethargy and convulsions without evidence of an infectious process. Laboratory examination reveals hyperammonemia with alkalosis. Glutamine, citrulline and arginine are found elevated in the blood. The enzyme that is most likely deficient in this patient is:
- A.
Arginase
- B.
Carbamoyl-phosphate synthetase I
- C.
Arginosuccinate lyase
- D.
Ornithine transcarbamoylase
- E.
Arginosuccinate synthetase
- 19.A newborn with acute encephalopathy is found to have hypoglycemia and ketonuria. Plasma leucine, isoleucine and valine are markedly elevated, but plasma ammonia is normal. This is most likely a case of:
- A.
Ornithine transcarbamoylase deficiency
- B.
Long-chain acyl-CoA dehydrogenase deficiency
- C.
Methylmalonic acidemia
- D.
Vitamin B12 deficiency
- E.
Maple syrup urine disease
- 20.A nurse notices that the urine produced by a newborn infant has the odor of maple syrup. Which of the following pathways is most likely defective?
- A.
Metabolism of branched-chain amino acids
- B.
Metabolism of aromatic amino acids
- C.
Metabolism of sulfur-containing amino acids
- D.
Transformation of carbohydrates to amino acids
- E.
One-carbon transfer reactions
- 21.A 1-year-old child with mental retardation has a deficiency of dihydrobiopterin reductase. This enzyme deficiency leads to an increased blood level of:
- A.
Tyrosine
- B.
Methionine
- C.
Glucocerebroside
- D.
Glycine
- E.
Phenylalanine
- 22.A.B. is transferred into the emergency room of a local hospital. Upon investigation high levels of glutamate:oxaloacetate transaminase (GOT) and glutamate:pyruvate transaminase (GPT) are found in his serum. His problems probably involve the following organ:
- A.
Heart
- B.
Brain
- C.
Liver
- D.
Muscle
- E.
Lungs
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